PROGRESSION OF AORTIC DILATATION AND THE BENEFIT OF LONG-TERM BETA-ADRENERGIC-BLOCKADE IN MARFANS-SYNDROME
PROGRESSION OF AORTIC DILATATION AND THE BENEFIT OF LONG-TERM BETA-ADRENERGIC-BLOCKADE IN MARFANS-SYNDROME
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DOI：
10.1056/nejm199405123301902
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发表时间：
1994-05-12
影响因子：
158.5
通讯作者：
PYERITZ, RE
中科院分区：
文献类型：
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作者：
SHORES, J;BERGER, KR;PYERITZ, RE
背景马凡氏综合征的主动脉根部进行性扩大，这种扩大与主动脉瓣返流和夹层有关。β-肾上腺素能阻滞剂的长期治疗，通过减少冲动（即，主动脉根部的压力变化率）的左心室射血和心率，可以保护主动脉根部。我们对患有典型马凡氏综合征的青少年和成人患者（32名治疗患者和38名未治疗[对照]患者）进行了一项普萘洛尔的开放标签、随机试验。主动脉根部尺寸和临床终点（主动脉瓣返流、主动脉夹层、心血管手术、充血性心力衰竭和死亡）在对照组中平均监测9.3年，在治疗组中平均监测10.7年。按意向治疗原则纳入分析。普萘洛尔的剂量个体化;平均（+/-SE）剂量为212+/-68 mg/天。治疗组中反映扩张率的牙根尺寸的回归线的平均斜率显著低于对照组（每年0.023 vs. 0.084，P
Background. The aortic root enlarges progressively in Marfan's syndrome, and this enlargement is associated with aortic regurgitation and dissection. Longterm treatment with beta-adrenergic blockade, by reducing the impulse (i.e., the rate of pressure change in the aortic root) of left ventricular ejection and the heart rate, may protect the aortic root.Methods. We conducted an open-label, randomized trial of propranolol in adolescent and adult patients with classic Marfan's syndrome (32 treated and 38 untreated [control] patients). Aortic-root dimensions and clinical end points (aortic regurgitation, aortic dissection, cardiovascular surgery, congestive heart failure, and death) were monitored for an average of 9.3 years in the control group and 10.7 years in the treatment group. All 70 patients were included in the analysis according to the intention-to-treat principle.Results. The dose of propranolol was individualized; the mean (+/-SE) dose was 212+/-68 mg per day. The mean slope of the regression line for the aortic-root dimensions, which reflect the rate of dilatation, was significantly lower in the treatment group than in the control group (0.023 vs. 0.084 per year, P